An Unusual Case of Hypoproteinemia in Childhood: Keep in Mind Trichobezoar
Autor: | Bruno Noccioli, Sandra Trapani, Massimo Resti, Alessandra Montemaggi, Salvatore Grosso, Mariangela Stinco |
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Jazyk: | angličtina |
Rok vydání: | 2020 |
Předmět: |
medicine.medical_specialty
trichobezoar medicine.medical_treatment Case Report 030204 cardiovascular system & hematology oedema Gastroenterology Pediatrics 03 medical and health sciences Hypoproteinemia 0302 clinical medicine Rapunzel Syndrome 030225 pediatrics Internal medicine Laparotomy medicine Rapunzel syndrome Enteropathy Trichophagia protein-losing enteropathy Past medical history business.industry Protein losing enteropathy lcsh:RJ1-570 lcsh:Pediatrics medicine.disease hypoproteinemia Intestinal malrotation Pediatrics Perinatology and Child Health business |
Zdroj: | Frontiers in Pediatrics Frontiers in Pediatrics, Vol 8 (2020) |
ISSN: | 2296-2360 |
Popis: | Protein-losing enteropathy (PLE) is a rare condition characterized by protein loss through the gastrointestinal tract, leading to hypo-proteinemia. Patients may be asymptomatic or present with variety of complications of hypoproteinemia (e.g., oedema, ascites, pleural, and cardial effusions). We describe a case report of a young girl suffering from behavioral disorder since childhood who presented with generalized oedema, hypoproteinaemia, and microcytic hypochromic anemia. In addition, the girl had an intervention for jejunal atresia and intestinal malrotation in her past medical history. Upper gastrointestinal endoscopy revealed a trichobezoar extending from stomach into the small bowel, thus classified as Rapunzel Syndrome (RS), causing mechanical obstruction of intestinal lumen and intestinal lymphatic drainage resulting in a protein-losing enteropathy (PLE). Trichobezoar was successfully removed by a surgical laparotomy resulting in resolution of symptoms and normalization of biochemical parameters. Possibly, previous surgery might have had an influence on intestinal dysmotility and trichobezoar formation. PLE is a very rare presenting symptom of RS, developing as result of intestinal obstruction caused by large trichobezoars. RS has to be considered in patients, especially adolescents, suffering from behavior disorder as trichotillomania and trichophagia. Surgical removal and nutritional supplementation are the gold treatment of large trichobezoar. |
Databáze: | OpenAIRE |
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